가부키 증후군(Kabuki syndrome) 환자의 치과적 관리의 치험례

DENTAL MANAGEMENT OF A PATIENT WITH KABUKI SYNDROME : A CASE REPORT

본 증례는 가부키 증후군 환아의 상악 좌측 제2유구치의 심한 우식으로 인하여 좌측 협부 종창이 관찰되었고, 물리적 속박 하에 절개 및 배농술을 시행 후 유구치는 발거되었다. 이후 전신마취 하에 남은 우식치아의 치료가 진행되었다. 가부키 증후군은 환자에 따라 다양한 의학적 소견을 보이므로 전신마취 하 치과 치료가 계획 될 경우, 의과와의 협진 및 철저한 치료계획이 필수적이다.

Kabuki syndrome is characterized by typical facial features (elongated palpebral fissures with eversion of the lateral third of the lower eyelid; arched and broad eyebrows; short columella with depressed nasal tip; large, prominent, or cupped ears), minor skeletal anomalies, persistence of fetal fingertip pads, mild to moderate intellectual disability, and postnatal growth deficiency. A 6-year-old male with kabuki syndrome was referred from the local dental clinic for left facial swelling and dental caries on all primary molars. He was treated for acute periapical abscess with incision and drainage under physical restraint, and left maxillary second primary molar was extracted. Other caries treatment was performed under general anesthesia. As the syndrome involves many different medical problems, special cares should be considered. Dental treatment should be carried out in comprehensive consultation system.